Article
Defining function of wild-type and three patient-specific TP53 mutations in a zebrafish model of embryonal rhabdomyosarcoma.
eLife - 2 Jun 2023
Chen Jiangfei, Baxi Kunal, Lipsitt Amanda E, Hensch Nicole Rae, Wang Long, Sreenivas Prethish, Modi Paulomi, Zhao Xiang Ru, Baudin Antoine, Robledo Daniel G, Bandyopadhyay Abhik, Sugalski Aaron, Challa Anil K, Kurmashev Dias, Gilbert Andrea R, Tomlinson Gail E, Houghton Peter, Chen Yidong, Hayes Madeline N, Chen Eleanor Y, Libich David S, Ignatius Myron S
Abstract excerpt
In embryonal rhabdomyosarcoma (ERMS) and generally in sarcomas, the role of wild-type and loss- or gain-of-function TP53 mutations remains largely undefined. Eliminating mutant or restoring wild-type p53 is challenging; nevertheless, understanding p53 variant effects on tumorigenesis remains central to realizing better treatment outcomes. In ERMS, >70% of patients retain wild-type TP53, yet mutations when present...
Topics
- Animals
- Carcinogenesis
- Cerebellar Neoplasms
- Mutation
- Proto-Oncogene Proteins p21(ras)
- Rhabdomyosarcoma, Embryonal
- Tumor Suppressor Protein p53
- Zebrafish